Whether hypertrophic cardiomyopathy  is diagnosed in individuals without symptoms or diagnosed during a family screening, a number of measures may be recommended. Typically, this means limiting physical activity and exercise to aerobic exercises and non-competitive sports that are not pursued to the point of either exhaustion of dehydration. Weightlifting and other isometric exercises should generally be avoided.

Hypertrophic cardiomyopathy often occurs in children, teens or young adults. This group tends to be more active by nature. The American Heart Association recommends that children and young adults should be evaluated by their physicians specifically to determine which recreational sports are safe and which physical activities should be avoided.

If people are not experiencing any symptoms, then a cardiologist might recommend only these activity restrictions and regular follow-up visits. An echocardiogram would typically be done at each of these visits. A search for electrical disturbances of the heart is imperative, even in an individual who does not exhibit symptoms.

If symptoms are present, or symptoms and signs of a problem are evident in testing, such as Holter monitoring, exercise stress testing, and other tests, the first course of treatment is often medications. These medications may be prescribed to relieve symptoms, such as shortness of breath, electrical disturbances of the heart, as well as to prevent heart failure. These medications include:

If medication is not successful, a permanent pacemaker may be considered. A pacemaker controls the heartbeat by pacing the heart at a slightly faster rate than the patient’s natural heartbeat. This treatment may be successful in reducing the major symptoms, chest pain and shortness of breath, in about 50 percent of patients. It is typically used in patients with hypertrophic obstructive cardiomyopathy (HOCM). By creating an abnormal pattern of electrical activity in the ventricle, the extent of the obstruction by the thickened muscle can be reduced. Pacemakers may not be as effective in reducing symptoms of hypertrophic cardiomyopathy as other forms of treatment.

In about five percent of cases, the blockage created by the heart’s enlargement must be reduced by surgical means. One procedure used is a septal myomectomy. During this surgery, the physician gains access to the left ventricle through the aortic valve. Once inside the heart, the physician removes part of the thickened septum (the muscular wall between the left and right ventricles). Recent research has shown that myomectomies are very effective at extending the life of patients who undergo the procedure. More than 75 percent of patients show some long-term improvement after this procedure.

In some cases, the mitral valve may need to be repaired or replaced at the same time. A mitral valve replacement may allow for the reduction of a severe leakage, called mitral regurgitation, which can contribute to symptoms of heart failure in this situation.

A relatively new procedure called septal alcohol ablation is also available for people with hypertrophic obstructive cardiomyopathy. This catheter-based procedure involves the destruction of certain heart cells with ethanol (alcohol) to shrink the heart muscle and increase the space for blood to flow. The ethanol can be placed in a specific branch of one of the heart’s arteries that provides blood to the septum. Results have been promising, showing long-term benefit as well as shorter recovery periods compared to traditional surgery. One common complication, however, has been the development of heart block, requiring implantation of a pacemaker. But researchers are already seeing a decrease in the rate of complications as the technique has evolved. This procedure may be recommended for patients who cannot withstand the rigors of surgery, but not all patients are candidates for this treatment. Research continues to evaluate the long-term effectiveness and safety of septal alcohol ablation.

Because a hypertrophic cardiomyopathy can cause potentially fatal arrhythmias, an electrophysiology study may be performed to determine whether an internally implantable cardioverter defibrillator (ICD) is necessary. In an electrophysiology study, the physician feeds a thin tube (catheter) through a blood vessel (usually a vein to the right ventricle. There, the physician tries to create a very fast heart rhythm (ventricular tachycardia) by introducing premature electrical impulses. If no ventricular tachycardia is created, then the patient is judged to be at lower risk of experiencing this condition in the future. On the other hand, if the ventricular tachycardia was elicited, an ICD may be necessary. Those with a family history of sudden cardiac death should be considered particularly at risk for an ICD.

An ICD may benefit patients who have experienced serious episodes of fainting (syncope), have been resuscitated from cardiac arrest or have experienced certain brief arrhythmias that occur without symptoms (a condition known as nonsustained ventricular tachycardia).

Because this disease appears to have a genetic component, there is no known way to prevent it. However, if there is family history of sudden cardiac death – even from no known cause – it is recommended that close relatives (e.g., siblings, children) see a cardiologist for a physical examination and echocardiogram to rule out or confirm early signs of disease.